Idiopathic pulmonary fibrosis (IPF) is a disease of unknown aetiology and uncertain pathogenesis, and there are no effective therapies. This is a devastating disease with an average survival of less than three years from the time of diagnosis.

Blood Test for Lung Fibrosis

Matrix metalloproteinases (MMPs) are zinc-dependent endopeptidases. Elevation of plasma matrix metalloproteinases (MMP1 and MMP7) may help to confirm a diagnosis of IPF and probably non-specific interstitial pneumonia (NSIP), and help to distinguish these diseases from hypersensitivity pneumonitis (HP), sarcoidosis, and perhaps other fibrotic lung diseases. However, a normal individual value does not exclude these diagnoses, as there is considerable overlap between levels of these MMPs in healthy people and patients with other chronic lung diseases. However, the plasma concentrations of MMP7 may be useful in following disease progression and even in facilitating early diagnosis. Source: PLoS Medicine, Creative Commons license.

Diagnosis

HRCT

A normal CXR does not rule out ILD. HRCT scan is the preferred imaging method for diagnosis of ILD. HRCT is false-negative in 12% of biopsy-proven ILD cases.

Biopsy can be useful in determine which patients may respond to CS. HRCT determines which patient needs lung biopsy. Biopsy can be done if there is ground glass appearance on HRCT with less than 25% reticular changes. If there are more than 25% reticular changes (more than 25% of lungs are inviolved), no biopsy is advised since CS will likely not be beneficial.

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